Pediatric Radiology: A novel plain abdominal radiograph sign to diagnose malrotation with volvulus Mahomed et al.

A novel plain abdominal radiograph sign to diagnose

malrotation with volvulus

Nataraja RM1, Mahomed AA1*

1. Department of Paediatric Surgery, Royal Alexandra Hospital for Sick Children, Brighton,UK

* Correspondence: Anies A Mahomed, Department of Paediatric Surgery, Royal Alexandra Hospital

for Sick Children, Eastern Road, Brighton, BN2 5B, UK
( Anies.Mahomed@bsuh.nhs.uk)

Radiology Case. 2010 May; 4(5):7-12 :: DOI: 10.3941/jrcr.v4i5.386

ABSTRACT
Journal of Radiology Case Reports

Malrotation with associated volvulus is a potentially lethal event for a

neonate. The gold standard for diagnosis is an upper gastrointestinal contrast
study. However this can delay the diagnosis and the timing of surgical
intervention. We present a novel abdominal radiographic sign; duodenal and

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gastric dilatation occurring in association with limited small bowel gas
confined to the right lower quadrant of abdomen and the total absence of
colonic air that is indicative of malrotation with associated volvulus. This
allows for an earlier diagnosis and expeditious surgery.

CASE SERIES

to ensure a favourable outcome. The investigation of choice of

INTRODUCTION
Malrotation of the small bowel is a common anomaly
occurring in 1 in 500 live births (1). It is a congenital failure of
the small bowel to complete the full 270o counter clockwise
rotation around the superior mesenteric artery (SMA). This
rotation, in normal fetal development, is achieved by three 90 o
counter clockwise rotations. The first occurs prior to 6 weeks
of gestation when the duodenum rotates 90o counter clockwise
and lies to the right of the SMA. After the 6th week the midgut
herniates from the peritoneal cavity and the duodenum
undergoes a second 90o counter clockwise rotation resulting in
the duodenum lying posteriorly in relation to the SMA. Then
between the 10th and 12th week of gestation the midgut
returns to the peritoneal cavity and the duodenum undergoes
the final 90o counter clockwise rotation so that the duodeno-
jejunal flexure lies to the left of the midline.
Malrotation when associated with a volvulus can result in
severe consequences such as short bowel syndrome, infarction
requiring bowel resection or even death. Paediatric surgical
trainees are taught to beware the neonate or infant with green-
coloured vomitus, and intestinal malrotation should always be
excluded if there are concerns (2). The prompt investigation
and surgical intervention of these neonates and infants is vital
a possible malrotation with associated volvulus in a stable
neonate is the upper gastrointestinal tract contrast study (3).
This can however potentially lead to a delay in surgical
intervention and therefore a worse outcome. In this article we
present a novel combination of abdominal radiographic
features, namely; duodenal and gastric dilatation occurring in
association with limited small bowel gas confined to the right
lower quadrant of abdomen and the total absence of colonic
air, which in newborn neonates indicates a malrotation with
volvulus and may negate the need for further investigations.
CASE DESCRIPTIONS
The first patient presented within a few hours of birth with
bile stained vomiting to the neonatal unit. The neonate was
delivered by normal vaginal delivery, required no post-natal
resuscitation, had normal APGAR scores and was also a term-
delivery. The initial clinical assessment revealed a well
neonate with a non-distended and non-tender abdomen. Initial
serological investigations revealed no abnormalities and no
acidosis or raised lactate levels. The initial plain abdominal
radiograph (Figure 1) confirmed duodenal dilatation (arrow 1),

Radiology Case. 2010 May; 4(5):7-12 7

 Pediatric Radiology: A novel plain abdominal radiograph sign to diagnose malrotation with volvulus
gas in proximal small bowel confined to the right of the
midline (arrow 2) and absent colonic air.
The second patient was also a term delivery, delivered in a
good condition with a normal postnatal examination and
serological investigations. There was an antenatal diagnosis of
possible small bowel obstruction and the immediate placement
of a nasogastric tube confirmed bilious aspirates. The antenatal
ultrasound report but not the images were available for review.
The postnatal plain abdominal radiograph (Figure 2)
confirmed the duodenal dilatation with gas in small bowel
confined to right hand side. No colonic gas was evident. The
transverse soft tissue shadow lying to the left of the midline
(arrowed) is the umbilical cord stump.
The third patient presented in a similar fashion to the first
neonate. The initial plain abdominal radiograph revealed the
same combination of radiological features (Figure 3). In this
case, however, the proximal small bowel dilatation was more
impressive than the other two cases.
Journal of Radiology Case Reports
Surgical intervention, in all of the three neonates,
confirmed the diagnosis of malrotation with volvulus and a
successful Ladd's procedure was performed. In all the patients
the laparotomy revealed viable bowel and there was no
indication for either bowel resection or stoma formation. All
of the patients underwent an uneventful post-operative course
and full enteral feeds were established with a discharge from
the neonatal unit within the first week of life.
DISCUSSION
Malrotation with associated volvulus can be a catastrophic
event for a newborn neonate. The investigation of choice, in a
stable infant is an upper gastrointestinal tract contrast study
(3). A normal duodenojejunal flexure (DJF) is seen to the left
of the spine and at the level of the pylorus (Figure 4).
Abnormalities seen with malrotation include; an abnormally
placed DJF, spiral or "corkscrew" distal duodenum, and
proximal jejunum located on the right in the abdominal cavity
(Figure 5) (4).
There are various other conditions that should be
considered on an abdominal radiograph when managing a
neonate who presents with bilious vomiting and duodenal
obstruction. These include duodenal atresia, the presence of a
duodenal web and also an annular pancreas.
In an otherwise well child, duodenal atresia has a typical
abdominal radiographic sign, the "double bubble". This
appearance is secondary to a distended first part of duodenum
and an air filled stomach. The absence of air distal to the
second part of duodenum distinguishes this condition from a
malrotation with volvulus or other conditions causing a partial
duodenal obstruction (Figure 6).
The duodenal lumen may also be partially obstructed by
the presence of a duodenal web, diaphragm or intraluminal
diverticulum. This results in the appearance of a dilated first
Radiology Case. 2010 May; 4(5):7-12
Mahomed et al.
part of the duodenum, distal air in the jejunum and a normally
placed duodenojejunal flexure (Figure 7). The distal jejunal air
would have a normal distribution compared to the confinement
to the right in our series of neonates. A duodenal web may also
be demonstrated with an upper gastrointestinal contrast study.
The abnormal duodenum can be demonstrated with a dilated
1st and 2nd part, the stomach may also be dilated but there will
be normal orientation of the rest of the small bowel and colon
(Figure 8).
Other rare causes of complete or incomplete duodenal
obstruction include the presence of an annular pancreas and
duodenal stenosis. With an annular pancreas, the second part
of the duodenum is obstructed by a ring of pancreatic tissue
arising from the head of the pancreas. This ring of tissue
encircles the duodenum either completely or partially resulting
in variable degrees of obstruction. Duodenal stenosis usually
has a delayed presentation with episodes of aspiration,
vomiting and failure to thrive.
There have been recent advances in ultrasound diagnosis
and features of malrotation include an abnormal relationship of
the superior mesenteric artery and vein and the classic
whirlpool sign of mesenteric pedicle twisting (5). The use of
this specialised technique remains, however, operator
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dependent. It is therefore not feasible as a routine method of
investigation even in some tertiary paediatric surgical centres.
There are various plain radiograph signs that have been
described in the literature for a paediatric malrotation with
associated volvulus. These include a gasless abdomen, a
"whirled" appearance secondary to volvulus, separation of
adjacent bowel loops with "tubular" appearance secondary to
ischaemia and also a normal gas pattern (4,6-8). It has also
been suggested that there is no clinical value of a plain
abdominal radiograph (8). We have, on the contrary, presented
in this article 3 cases of neonates who presented with signs of
intestinal obstruction with operative finding of malrotation
with volvulus. All the supine radiographs were taken within
the first 24 hours of life. Consistent features in all of these
were; duodenal and gastric dilatation occurring in association
with limited small bowel gas confined to the right lower
quadrant of abdomen and the total absence of colonic air.
These three neonates were subjected to a laparotomy and were
found to have malrotation with volvulus but with viable bowel.
All underwent a successful Ladd's procedure which involved, a
derotation of the bowel, division of Ladd's bands, widening of
the vascular pedicle and appendicectomy. In addition,
intubation of the duodenum was carried out to exclude a web.
This peculiar radiological appearance is secondary to a
malrotation with associated volvulus causing a partial
duodenal obstruction leading to proximal dilatation. Duodenal
distension (as noted in figures 1-3) is variable and dependant
on the longevity and the degree of obstruction. Irrespective of
this, duodenal occlusion would appear to be partial, and a
small amount of air does pass through into the small bowel
which is visible to the right of the midline. On initial post natal
abdominal X ray no air appears to pass distally into the colon
hence its imperceptibility. As the air makes its way into the
colon it is likely that this feature would transform over time.
8
 Pediatric Radiology: A novel plain abdominal radiograph sign to diagnose malrotation with volvulus Mahomed et al.

This constellation of radiographic features has hitherto not
been described and in combination indicates a volvulus. This
may be an indication for immediate laparotomy and a Ladd's
procedure without requirement for an upper gastrointestinal
contrast study. There is a need, however, for a larger
investigation of neonates who present with bilious vomiting to
determine the sensitivity and specificity of this constellation of
radiographic features in malrotation with volvulus. Although
the absence of this sign does not exclude malrotation with
volvulus and its associated sequalae, its presence should
ensure there is no delay in surgical intervention by avoiding
unnecessary investigations with the associated hazard of
radiation exposure.
5. John SD. Imaging of acute Abdominal Emergencies in
Infants and children. Curr Probl Pediatr (2001) 31:315-53.
6. Daneman A. Malrotation: The balance of evidence. Pediatr
Radiol (2009) 39 (Suppl 2): S164-S166.
7. Lampl B, Levin TL, Berdon WE, Cowles RA. Malrotation
and midgut volvulus; a historical review and current
controversies in diagnosis and management. Pediatr Radiol
(2009) 30:359-366.
8. Applegate KE. Evidence-based diagnosis of malrotation and
volvulus. Pediatr Radiol (2009) 39 (Suppl 2):S161-S163.

CONCLUSION FIGURES

Combination of duodenal dilatation, gas in proximal small

bowel confined to theTEACHING
right of the POINT
midline and absent colonic
air on an initial plain abdominal radiograph of a neonate aids
Journal of Radiology Case Reports

expeditious diagnosis of malrotation with volvulus. This

allows earlier surgical intervention and an improved outcome.
A larger series is required to ascertain the sensitivity and
specificity of this sign. For the meantime, for all suspected

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cases of malrotation the gold standard of radiological
investigation remains the upper gastrointestinal contrast study.

TEACHING POINT

Malrotation with associated volvulus is a potentially lethal

event for a newbornTEACHING POINT
neonate presenting with bilious vomiting.
An abnormally placed duodeno-jejunal flexure demonstrated
on an upper gastrointestinal contrast study, or the presence of
duodenal and gastric dilatation with limited small bowel gas
confined to the right lower quadrant of abdomen and the total
absence of colonic air on an early post natal X ray is indicative
of malrotation with volvulus and requires prompt diagnosis
and treatment.

REFERENCES
Figure 1: Initial AP abdominal radiograph of 1 day old male
1. Miyakoshi K, Ishimoto H, Tanigaki S, et al. Prenatal
neonate presenting within a few hours of birth with bile stained
diagnosis of midgut volvulus by sonography and magnetic
vomiting. An initial radiograph confirmed duodenal dilatation
resonance imaging. Am J Perinatol (2001) 18:447-450.
(arrow 1), gas in proximal small bowel confined to the right of
the midline (arrow 2) and absent colonic air.
2. Millar A, Rode H, Cywes S. Malrotation and volvulus in
infancy and childhood. Semin Pediatr Surg (2004) 12:229-
236.

3. Sizemore AW, Rabbani KZ, Ladd A et al. Diagnostic

performance of the upper gastrointestinal series in the
evaluation of children with clinically suspected malrotation/
Pediatric Radiol (2008) 38:518-528.

4. Strouse PJ. Disorders of intestinal rotation and fixation

("malrotation"). Pediatr Radiol (2004) 34:837-851.

Radiology Case. 2010 May; 4(5):7-12 9

 Pediatric Radiology: A novel plain abdominal radiograph sign to diagnose malrotation with volvulus Mahomed et al.

Figure 4: Abdominal upper gastrointestinal Gastrograffin

contrast study of a three day old term male neonate who
presented with bilious vomiting. A normal duodenojejunal
flexure (DJF) is demonstrated on the left of the spine and at
Journal of Radiology Case Reports

the level of the pylorus (arrow). The classic "C" shape of the
duodenum is also demonstrated.

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Figure 2: AP abdominal radiograph of 1 day old female
neonate with ultrasound antenatal diagnosis of possible small
bowel obstruction. Duodenal dilatation is noted with gas in
small bowel confined to right hand side. No colonic gas was
evident. The transverse soft tissue shadow lying to the left of
the midline (arrowed) is the umbilical cord stump.

Figure 3: AP abdominal radiograph of a 1 day old male

neonate who presented with bile stained vomiting. Gastric
(arrow 1) and some duodenal dilatation (arrow 2) was revealed
with more impressive small bowel shadows confined to the
right side of the abdomen (arrow 3). There is an absence of
distal small bowel or colonic air.

Radiology Case. 2010 May; 4(5):7-12 10

 Pediatric Radiology: A novel plain abdominal radiograph sign to diagnose malrotation with volvulus Mahomed et al.
Journal of Radiology Case Reports

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Figure 5: Upper gastrointestinal barium contrast study in a 4 day old female neonate who presented with bilious vomiting with
associated abdominal distension demonstrating features of malrotation with volvulus. The lateral view on the left demonstrates
the spiral configuration to the bowel just distal to the second part of duodenum secondary to the volvulus (arrow). The AP view
on the right shows the proximal small bowel to be lying to the right of the midline. The classic "C" of duodenum with the DJ
flexure lying to the left of the midline is not seen.

Figure 6 (left): Abdominal radiograph of a 1 day old term

male neonate who presented with bilious vomiting secondary
to a duodenal atresia. The "double bubble" is seen with a
distended first part of duodenum (arrow 1) and air filled
stomach (arrow 2). The absence of air distal to the second part
of duodenum distinguishes this condition from a malrotation
with volvulus.

Radiology Case. 2010 May; 4(5):7-12 11

 Pediatric Radiology: A novel plain abdominal radiograph sign to diagnose malrotation with volvulus Mahomed et al.
Journal of Radiology Case Reports

Figure 7: Abdominal radiograph of a 1 day old male neonate
with a duodenal web. The web is demonstrated by a distended
proximal duodenum (arrow 1) and distal air present in the
jejunum (arrow 2). A coiled nasogastric tube is seen within the
stomach (arrow 3). The duodenojejunal junction also lies to
the left of the midline distinguishing it from malrotation with
associated volvulus (arrow 4).
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Figure 8: Upper gastrointestinal Niopam 360 contrast study in
a 1 day male old neonate with a duodenal web. There is an
abnormal duodenum demonstrated but with normal orientation
of the rest of the small bowel and colon. The duodenal web is
demonstrated by a distended stomach (arrow 1), 1st and 2nd
(arrow 2 & 3) parts of the duodenum.

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ABBREVIATIONS www.radiologycases.com/index.php/radiologycases/article/view/386

DJF = duodenojejunal flexure

SMA = superior mesenteric artery Interactivity
This publication is available as an interactive article with
scroll, window/level, magnify and more features.
Available online at www.RadiologyCases.com

KEYWORDS
Published by EduRad
Malrotation, volvulus, abdominal radiograph

www.EduRad.org

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